How do you choose first or second-line systemic therapy for fibrolamellar hepatocellular carcinoma?

Fibrolamellar carcinoma is histologically unique from hepatocellular carcinoma. The disease is correctly called fibrolamellar carcinoma, and not fibrolamellar hepatocellular carcinoma, or hepatocellular carcinoma fibrolamellar variant. Even though the latter may be permissive if the lack of cirrhosis is stated (Semin Diagn Pathol. 2017 Mar;34(2):146-152) plus more.

This is important to avoid treating patients with fibrolamellar carcinoma by default like patients with hepatocellular carcinoma. This is critical, especially in the advent of so many new therapies for hepatocellular carcinoma that do not apply to fibrolamellar carcinoma. For example, atezolizumab plus bevacizumab is not a validated therapy for fibrolamellar carcinoma. Chemothera...